Primary Paediatric Renal Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature.

Ewing sarcoma/primitive neuroectodermal tumor (PNET) is a high-grade malignant neoplasm commonly affecting bones of the thoracic region. Primary Ewing sarcoma/PNET of the kidney is exceptional; it commonly affects young adults and is rarely reported in children. Here we describe a case of renal PNET in a 10-year-old girl who presented at our institute with haematuria and abdominal lump. Computer tomography scan revealed a huge mass in the right kidney. Computer tomography-guided aspiration from the mass showed cytomorphological features of a small blue round cell tumor. The patient underwent right radical nephrectomy. Histopathology with supplementary immunohistochemistry confirmed the diagnosis of PNET. She has been receiving treatment with adjuvant chemotherapy post-surgery and is currently disease free. Primary renal PNET is a distinct and rare entity. This tumor is very aggressive with low survival rate, even with a multi-disciplinary approach. We reported the case because it is rare in children, poses diagnostic challenges, is aggressive in behaviour and responds poorly to treatment.

Ectopic Paratubal Adrenal Cell Rest Associated with Mucinous Cystadenoma of Ovary.

Ectopic adrenal cortex is a rare entity. Usually found in male children; commonly located around kidney, retroperitoneum, spermatic cord and para-testicular region. Rarely, adults with heterotopic adrenal glands are described. Incidence in females is very less; though sometimes detected accidentally in hysterectomy specimens. We describe a case of ectopic adrenal cortical cell in paratubal region in a patient with mucinous cyst adenoma of ovary. A 26-year-old female presented with complains of menstrual irregularities and abdominal discomfort for 6 months. Investigations suggested a right ovarian cyst. Right ovarian cystectomy with partial salpingectomy was performed; histopathology revealed mucinous cyst adenoma. Sections from tube showed presence of ectopic adrenal cortical rest in the paratubal region, incidentally discovered on microscopy. We present this case because of its rarity in females, interesting presentation with another unrelated gynaecological pathology, its potentiality for malignant transformation and possible complications.

Spindle-cell ameloblastic carcinoma of the maxilla with adenoid cystic carcinoma-like areas: A new variant?

Ameloblastic carcinoma is a rare aggressive malignancy of odontogenic epithelial origin. The disease has been reported to afflict people over a wide range of age group without any sex predilection. It is characterized by rapid growth and destruction of overlying bones along with involvement of adjacent soft tissues. The overall prognosis of this cancer is poor despite radical surgery and radiotherapy. Owing to the rarity of this lesion, complete data on the biological behavior and response to different treatment modalities are still lacking. The authors hereby report a case of this uncommon tumor arising from left maxilla in a 60-year-old Indian male. Histopathological examination of the tumor showed foci of spindling intermixed with large areas of tumor cells arranged in cribriform pattern and having luminal eosinophilic material which was reminiscent of adenoid cystic carcinoma. A final diagnosis was achieved after immunohistochemical study. These unusual findings prompted this case report.

Eosinophilic granuloma of the mandible: a diagnostic dilemma.

Eosinophilic granuloma (EG) is a rare histiocytic disorder resulting from clonal proliferation of Langerhans cells. It accounts for less than 1% of all osseous neoplasms and has a predilection for involving the axial skeleton. Although suspicion of the disease may arise from clinical features and radiographic demonstration of destructive bone lesions, it is still difficult to make a correct diagnosis without proper pathological evaluation. This is more evident when common differentials mimicking EG, both clinically and radiologically, need to be ruled out. This report describes a case of unifocal EG of the mandible occurring in a 4-year-old boy whose initial presentation led to confusion between osteomyelitis, primary bone tumour and lymphoma. A final diagnosis of EG was established after histopathological examination of the biopsy specimen.

The significance of microbiopsies in cytological smears.

BACKGROUND: The role of fine needle aspiration cytology (FNAC) in the diagnosis of malignant lesions is well documented. Conventionally obtained fine needle aspiration (FNA) smears frequently contain well preserved viable intact tissue fragments (microbiopsies). AIMS: The present study was aimed at evaluating the microbiopsies and carrying out further cyto-histopathologic correlation in order to assess what additional information they could provide. MATERIALS AND METHODS: A total of 116 FNA smears from clinically suspected malignant lesions were examined, of which 81 smears (70.0%) contained representative tissue fragments of the tumors. Histopathological details were available in 75 cases. Immunocytochemistry (ICC) was applied in selected smears as and when required. RESULTS: Tumors in which microbiopsies aided in diagnosis chiefly included soft tissue malignancies (12 cases), typed into malignant peripheral nerve sheath tumor, fibrosarcoma, spindle cell sarcoma and malignant melanoma; lung tumors (14 cases) classified as small cell carcinoma, large cell carcinoma, squamous carcinoma and adenocarcinoma; breast tumors (08 cases), typed into ductal, medullary and lobular carcinoma; and lymph node metastasis (19 cases) from primaries in lung, larynx and thyroid. The cyto-histopathologic concordance was raised from 81.2% in the absence of microbiopsies to 93.2% in their presence. CONCLUSIONS: FNA smears containing microbiopsies are of ample help in establishing firm diagnosis, tumor typing, and predicting possible primary sites in metastatic tumors which were not possible by cytology alone. Hence, this technique can be utilized to enhance the diagnostic accuracy of FNAC, if put into practice in evaluation of routine cytology smears, without increasing any financial burden on patients.

Myxoid neurothekeoma: a rare soft tissue tumor of hand in a male toddler.

Neurothekeomas are rare benign neoplasms, typically occurring in young patients with a remarkable predilection for the female population. Patients usually present with a small nodule in different anatomical sites, commonly involving the face and the upper limb. We report a case of a three-year-old boy, who presented with a nontender nodule on the left thumb. Surgical biopsy and immunostaining confirmed the diagnosis as myxoid neurothekeoma. The rarity of this unusual skin tumor in a toddler prompted the following report.

Primary squamous cell carcinoma of the parotid gland: a rare entity.

Primary squamous cell carcinoma of the parotid gland is a rare aggressive malignancy. It is a rapidly advancing lesion which, if not recognised and treated early, results in high morbidity and mortality. Despite radical surgery and adjuvant radiotherapy, prognosis of this cancer continues to be poor. Careful clinical and histological examination is mandatory to differentiate this tumour from metastatic squamous cell carcinoma and other primary malignancies of the parotid. The authors hereby report the case of a 50-year-old male patient who presented with a progressively increasing, painless mass in parotid region of 6 months duration. An initial fine-needle aspiration cytology and subsequent histopathological examination confirmed that the tumour was squamous cell carcinoma. As no other primary source could be demonstrated in the patient, a final diagnosis of primary squamous cell carcinoma of parotid was offered. Currently the patient is on regular follow-up without any signs of recurrence.

Diagnosis of rosai-dorfman disease in elderly female on fine needle aspiration cytology: a case report.

Rosai-Dorfman disease (RDD) is a rare benign disorder of histiocytic proliferation that usually presents with bilateral cervical lymphadenopathy in children. We describe the case of a 50-year-old lady suffering from this disease who presented with generalized lymphadenopathy and a left sided chest wall lump. Fine needle aspiration cytology (FNAC) from all the lesions showed abundant benign histiocytes with lymphophagocytosis which was compatible with the diagnosis of RDD. This case is being reported for its rarity in presentation in an elderly female with both generalized nodal as well as extranodal manifestations.